DOI: https://doi.org/10.22141/2224-1485.6.62.2018.153930

Pulmonary hypertension associated with connective tissue diseases: a literature review focusing on diagnostic aspects

Ye.D. Yehudina, O.O. Khaniukov, I.Yu. Golovach, O.S. Kalashnykova

Abstract


Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTD). CTD may cause PH due to several mechanisms; pulmonary arterial hypertension (PAH), associated interstitial lung disease, neuromuscular disease, and/or sleep-disordered breathing leading to hypoxia, associated thromboembolic PH, and pulmonary venous hypertension due to left ventricular dysfunction. Recent studies have clarified that pulmonary hypertension associated with connective tissue diseases (CTD-PАH) has some distinctive clinical aspects from other PH, such as high prevalence, venous and cardiac involvement, less favourable outcomes, presence of DETECT algorithm, response to immunosuppression, existence of combined forms. Pressure in pulmonary artery can be measured on echocardiography, but the gold standard for the diagnosis is right heart catheterization. PАH-specific therapy in addition to immunosuppression is the most common treatment used for CTD-PАH patients, there are currently many controversial thoughts about the beginning and duration of these drug prescribing. In this review, we discuss the epidemiologic burden, pathogenesis, clinical presentation of CTD-PАH, focus physicians’ attention on the diagnostic criteria and algorithms of early disease detection and management of these patients.


Keywords


systemic connective tissue disease; pulmonary hypertension; mixed connective tissue disease; systemic sclerosis; systemic lupus erythrematosus; review

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